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MASTOCYTOSE
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From The Mastocytosis Chronicles, Volume 2, Number 1

The Usual Treatments By Olive Clayson

Mastocytosis is the general term for a variety of rare disorders in which there is a proliferation of mast cells in the skin, GI tract, or throughout the entire body.
No matter what type of Mastocytosis you have, the goal of treatment is to provide patient relief by either preventing the degranulation of those mast cells, or by blocking the effects of mast cell degranulation.

Degranulation, or the rupturing of a mast cell with the release of histamine, leukotrienes, and prostaglandin D2, can be triggered by a wide variety of things such as temperature changes and extremes, stress, alcohol, various foods and drugs, and even various smells.
It is the responsibility of the individual patient to be aware of all the things in their environment that cause them problems.

There is no known cure for Mastocytosis, so physicians must resort to treating the symptoms. The symptoms of this disease vary widely, and it has been rare to find two patients who have exactly the same set of symptoms. That is the primary reason why Mastocytosis patients often endure years of misdiagnosis before the cause of their symptoms is revealed.
Once Mastocytosis is diagnosed, the physician may recommend treatment based on trial and error, with the goal of finding the right combinations of drugs for the individual patient. Some of the most common treatments include:
  • H1 antihistamines such as chlorpheniramine maleate (found in many over-the-counter cold medications), and cyproheptadine hydrochloride (Periactine®), help with symptoms such as pruritus (itching), dermatographism, urticaria pigmentosa, and are often used in conjunction with epinephrine after particularly strong Mastocytosis attacks.
    Doxepin hydrochloride (Sinequan(R)), although officially classified as a tricyclic antidepressant, has a very powerful antihistamine effect and is frequently prescribed to control resistant symptoms.
    Antihistamines appear to compete with histamine for receptor sites on effector cells. If a cell receptor site is occupied by an antihistamine, it can't chemically interact with histamine, and therefore the chemical cycle is broken before the histamine can work to create a symptom.
    Nonsedating antihistamines, such as Hismanal(R) (astemizole) and Claritin(R) (loratadine), may also be helpful.
  • H2 antihistamines such as ranitidine hydrochloride (Zantac(R)), Famotidine (Pepcid(R)), or cimetidine hydrochloride (Tagamet(R)), treat pathological gastric hypersecretory conditions. They tie up different receptor sites than H1 antihistamines, called H2 receptors.
  • The most valuable drug for the emergency treatment of severe Mastocytosis symptoms is epinephrine.
    The vasoconstrictor effect of epinephrine directly counteracts the vasodilatation produced by histamine. Epinephrine quickly restores circulating blood volume and blood pressure, and quickly eliminates itching and swelling.
    Many Mastocytosis patients carry "bee sting kits" so they can inject epinephrine at the onset of a Mastocytosis attack, or use an epinephrine inhalation aerosol such as Primatene(R) Mist, which is good for laryngeal swelling and can possibly have systemic benefits. If alcohol is a Masto trigger for you, make sure the inhaler you choose is alcohol free. You may have to special order an alcohol free inhaler.
  • Cromolyn sodium (Gastrocrom®) is the only drug made specifically for Mastocytosis patients. It inhibits the release of histamine and leukotrienes from sensitized mast cells, and if mast cells don't degranulate, they are less likely to cause problems.
    Histamine stimulates gastric secretion and cause flushing of the skin, lowered blood pressure, and headaches, while leukotrienes are the chemical agents which trigger inflammation. Leukotrienes cause dermatographia, the painful red wheals raised when you scratch the skin of a Mastocytosis patient. They are also powerful bronchial constrictors and vasodilators, causing the bronchial distress and anaphylaxis often associated with Mastocytosis.
  • Nonsteroidal anti-inflammatory agents such as aspirin and similar NSAIDs are often used to directly inhibit the synthesis of prostaglandin D2 (PGD2). Steroidal anti-inflammatory agents such as prednisone don't inhibit PGD2 directly; rather they inhibit formation of arachidonic acid metabolites in many tissues by blocking phospholipase A2. Steroids indirectly prevent PGD2 formation by turning off the whole cascade of arachidonic acid metabolism. Prostaglandins, which are also released when mast cells degranulate, influence blood flow and gastrointestinal function and activity.
  • Interferon, a protein released by white blood cells to fight viral infections, is being tried to treat Mastocytosis systemically. Its primary side effects are flu-like symptoms and arthralgias.
  • PUVA therapy (photochemotherapy with psoralen, a plant derivative, and ultraviolet A irradiation) is the use of light and chemicals together to fight the disease in the skin.
. There are many treatments offered to Mastocytosis patients that are generally not approved by the medical establishment, or can be used in conjunction with traditional therapies.
Ketotifen has been shown to be helpful to some patients. Ketotifen, however, is not approved for use in the United States, but may be purchased in Canada and Mexico. Blue-green algae is used as a mineral supplement, a source of vitamin B12 §-carotene, protein, and amino acids. Some patients report that it has markedly helped with malabsorption and other GI symptoms. There are a variety of herbal medications that are either drunk as tea or injected. Acupuncture, therapeutic massage, and chiropractic manipulation can all offer some symptomatic relief.
A good rule of thumb is: don't take any drugs unless they are prescribed by your doctor. Any drug can cause severe reactions in Mastocytosis patients, and most drugs come with a handful of possible side effects and warnings, i.e., may impair mental alertness, cause confusion, diarrhea, nausea and vomiting.
Remember that treatments are usually based on trial and error, so a patient has to be aware of any effects a new therapy is having. It is the responsibility of the individual patient to be aware of all the things in their environment that cause them problems. Talk to your doctor before trying any nontraditional therapy. He may want to monitor you more closely when you are trying something new.
Mastocytosis is a rare disease, and not all physicians are aware of the standard treatments. To make the most of the medical help available, educate yourself and become a partner doctor in controlling your disease.

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